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Sleep Disorders in Patients with Choreic Syndromes

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Abstract

Purpose of Review

Patients with different types of choreic syndromes, specially those with Huntington’s (HD) and Wilson’s (WD) diseases, report frequent sleep complaints. This review focuses on the main findings of studies addressing the sleep features in these diseases, and other less frequent causes of chorea associated with sleep disorders, including a new syndrome described in the last decade associated with IgLON5 antibodies.

Recent Findings

Patients with HD and WD showed a bad quality of sleep and high frequency of insomnia and excessive daytime somnolence. WD patients also showed high scores on a specific scale for rapid eye movement sleep behavior disorders. HD and WD share decreased sleep efficiency and increased REM sleep latencies, percentage of sleep stage N1, and wake after sleep onset (WASO) among their polysomnographic features.

Summary

Patients with HD and WD showed a high prevalence of different sleep disorders. Patients with other causes of chorea, including neuroacanthocytosis, parasomnia with sleep breathing disorder associated with antibodies to IgLON5, Sydenham's chorea, and choreic syndromes associated to certain genetic mutations show sleep disorders as well.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Raggi A, Bella R, Pennisi G, Neri W, Ferri R. Sleep disorders in Parkinson’s disease: a narrative review of the literature. Rev Neurosci. 2013;24(3):279–91. https://doi.org/10.1515/revneuro-2013-0002.

    Article  PubMed  Google Scholar 

  2. Lajoie AC, Lafontaine AL, Kaminska M. The spectrum of sleep disorders in Parkinson’s disease: a review. Chest. 2021;159(2):818–27. https://doi.org/10.1016/j.chest.2020.09.099.

    Article  PubMed  Google Scholar 

  3. Jiménez-Jiménez FJ, Alonso-Navarro H, García-Martín E, Agúndez JAG. Sleep disorders in essential tremor: systematic review and meta-analysis. Sleep. 2020;43(9):zsaa039. https://doi.org/10.1093/sleep/zsaa039.

    Article  PubMed  Google Scholar 

  4. Jiménez-Jiménez FJ, Alonso-Navarro H, García-Martín E, Agúndez JAG. Sleep disorders in patients with essential tremor. Curr Neurol Neurosci Rep. 2021;21(5):23. https://doi.org/10.1007/s11910-021-01109-y.

    Article  PubMed  Google Scholar 

  5. Hibberd C, Charman T, Bhatoa RS, Tekes S, Hedderly T, Gringras P, Robinson S. Sleep difficulties in children with Tourette syndrome and chronic tic disorders: a systematic review of characteristics and associated factors. Sleep. 2020;43(6):zsz308. https://doi.org/10.1093/sleep/zsz308.

    Article  PubMed  Google Scholar 

  6. Jiménez-Jiménez FJ, Alonso-Navarro H, García-Martín E, Agúndez JAG. Sleep disorders in Tourette syndrome. Sleep Med Rev. 2020;53:101335. https://doi.org/10.1016/j.smrv.2020.101335.

    Article  PubMed  Google Scholar 

  7. Keenan L, Sherlock C, Bramham J, Downes M. Overlapping sleep disturbances in persistent tic disorders and attention-deficit hyperactivity disorder: a systematic review and meta-analysis of polysomnographic findings. Neurosci Biobehav Rev. 2021;126:194–212.

    Article  PubMed  Google Scholar 

  8. Blaty JL, DelRosso LM. Tourette disorder and sleep. Biomed J. 2022;45(2):240–9. https://doi.org/10.1016/j.bj.2022.01.002.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Jiménez-Jiménez FJ, Alonso-Navarro H, García-Martín E, Agúndez JAG. Sleep disorders and sleep problems in patients with Tourette syndrome and other tic disorders: current perspectives. Nat Sci Sleep. 2022;25(14):1313–31. https://doi.org/10.2147/NSS.S340948.

    Article  Google Scholar 

  10. •• Herzog-Krzywoszanska R, Krzywoszanski L. Sleep disorders in Huntington’s disease. Front Psychiatry. 2019;12(10):221. https://doi.org/10.3389/fpsyt.2019.00221. Review on sleep disorders in Huntington’s disease.

    Article  Google Scholar 

  11. •• Cochen De Cock V, Woimant F, Poujois A. Sleep disorders in Wilson’s disease. Curr Neurol Neurosci Rep. 2019;19(11):84. https://doi.org/10.1007/s11910-019-1001-4. Review on sleep disorders in Huntington’s disease.

    Article  PubMed  Google Scholar 

  12. •• Xu J, Deng Q, Qin Q, Vgontzas AN, Basta M, Xie C, Li Y. Sleep disorders in Wilson disease: a systematic review and meta-analysis. J Clin Sleep Med. 2020;16(2):219–30. https://doi.org/10.5664/jcsm.8170. Meta-analysis of sleep disorders in Wilson’s disease.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Walker FO. Huntington’s disease. Lancet. 2007;369(9557):218–28. https://doi.org/10.1016/S0140-6736(07)60111-1.

    Article  CAS  PubMed  Google Scholar 

  14. Roos RA. Huntington’s disease: a clinical review. Orphanet J Rare Dis. 2010;20(5):40. https://doi.org/10.1186/1750-1172-5-40.

    Article  Google Scholar 

  15. Ferreira JJ, Rodrigues FB, Duarte GS, Mestre TA, Bachoud-Levi AC, Bentivoglio AR, Burgunder JM, Cardoso F, Claassen DO, Landwehrmeyer GB, Kulisevsky J, Nirenberg MJ, Rosser A, Roth J, Seppi K, Slawek J, Furr-Stimming E, Tabrizi SJ, Walker FO, et al. An MDS evidence-based review on treatments for Huntington’s disease. Mov Disord. 2022;37(1):25–35. https://doi.org/10.1002/mds.28855.

    Article  PubMed  Google Scholar 

  16. Iakhno NN. Narusheniia nochnogo sna pri khoree Gentingtona [Disorders of nocturnal sleep in Huntington chorea]. Zh Nevropatol Psikhiatr Im S S Korsakova. 1985;85(3):340–6.

    CAS  PubMed  Google Scholar 

  17. Hansotia P, Wall R, Berendes J. Sleep disturbances and severity of Huntington’s disease. Neurology. 1985;35(11):1672–4. https://doi.org/10.1212/wnl.35.11.1672.

    Article  CAS  PubMed  Google Scholar 

  18. Taylor N, Bramble D. Sleep disturbance and Huntingdon’s disease. Br J Psychiatry. 1997;171:393. https://doi.org/10.1192/bjp.171.4.393c.

    Article  CAS  PubMed  Google Scholar 

  19. Videnovic A, Leurgans S, Fan W, Jaglin J, Shannon KM. Daytime somnolence and nocturnal sleep disturbances in Huntington disease. Parkinsonism Relat Disord. 2009;15(6):471–4. https://doi.org/10.1016/j.parkreldis.2008.10.002.

    Article  PubMed  Google Scholar 

  20. Neutel D, Tchikviladzé M, Charles P, Leu-Semenescu S, Roze E, Durr A, Arnulf I. Nocturnal agitation in Huntington disease is caused by arousal-related abnormal movements rather than by rapid eye movement sleep behavior disorder. Sleep Med. 2015;16(6):754–9.

    Article  PubMed  Google Scholar 

  21. Baker CR, Domínguez DJF, Stout JC, Gabery S, Churchyard A, Chua P, Egan GF, Petersén Å, Georgiou-Karistianis N, Poudel GR. Subjective sleep problems in Huntington’s disease: a pilot investigation of the relationship to brain structure, neurocognitive, and neuropsychiatric function. J Neurol Sci. 2016;15(364):148–53. https://doi.org/10.1016/j.jns.2016.03.021.

    Article  Google Scholar 

  22. Ogilvie AC, Nopoulos PC, Schultz JL. Sleep disturbances by disease type and stage in Huntington’s disease. Parkinsonism Relat Disord. 2021;91:13–8. https://doi.org/10.1016/j.parkreldis.2021.08.011.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Tanigelov-Yusim N, Barer Y, Martinec M, Siadimas A, Roumpanis S, Furby H, Goldshtein I, Jan A, Coloma PM. Huntington’s disease in Israel: a population-based study using 20 years of routinely-collected healthcare data. J Huntingtons Dis. 2021;10(4):469–77. https://doi.org/10.3233/JHD-210500.

    Article  Google Scholar 

  24. Arnulf I, Nielsen J, Lohmann E, Schiefer J, Wild E, Jennum P, Konofal E, Walker M, Oudiette D, Tabrizi S, Durr A. Rapid eye movement sleep disturbances in Huntington disease. Arch Neurol. 2008;65(4):482–8. https://doi.org/10.1001/archneur.65.4.482.

    Article  PubMed  Google Scholar 

  25. Aldaz T, Nigro P, Sánchez-Gómez A, Painous C, Planellas L, Santacruz P, Cámara A, Compta Y, Valldeoriola F, Martí MJ, Muñoz E. Non-motor symptoms in Huntington’s disease: a comparative study with Parkinson’s disease. J Neurol. 2019;266(6):1340–50. https://doi.org/10.1007/s00415-019-09263-7.

    Article  PubMed  Google Scholar 

  26. Furby H, Siadimas A, Rutten-Jacobs L, Rodrigues FB, Wild EJ. Natural history and burden of Huntington’s disease in the UK: a population-based cohort study. Eur J Neurol. 2022;29(8):2249–57. https://doi.org/10.1111/ene.15385.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Maffi S, Scaricamazza E, Migliore S, Casella M, Ceccarelli C, Squitieri F. Sleep quality and related clinical manifestations in Huntington disease. J Pers Med. 2022;12(6):864. https://doi.org/10.3390/jpm12060864.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Aziz NA, Anguelova GV, Marinus J, Lammers GJ, Roos RA. Sleep and circadian rhythm alterations correlate with depression and cognitive impairment in Huntington’s disease. Parkinsonism Relat Disord. 2010;16(5):345–50. https://doi.org/10.1016/j.parkreldis.2010.02.009.

    Article  PubMed  Google Scholar 

  29. Lazar AS, Panin F, Goodman AO, Lazic SE, Lazar ZI, Mason SL, Rogers L, Murgatroyd PR, Watson LP, Singh P, Borowsky B, Shneerson JM, Barker RA. Sleep deficits but no metabolic deficits in premanifest Huntington’s disease. Ann Neurol. 2015;78(4):630–48. https://doi.org/10.1002/ana.24495.

    Article  PubMed  PubMed Central  Google Scholar 

  30. Piano C, Imperatori C, Losurdo A, Bentivoglio AR, Cortelli P, Della MG. Sleep-related modifications of EEG connectivity in the sensory-motor networks in Huntington disease: an eLORETA study and review of the literature. Clin Neurophysiol. 2017;128(7):1354–63. https://doi.org/10.1016/j.clinph.2016.11.019.

    Article  PubMed  Google Scholar 

  31. Piano C, Della Marca G, Losurdo A, Imperatori C, Solito M, Calandra-Buonaura G, Provini F, Cortelli P, Bentivoglio AR. Subjective assessment of sleep in Huntington disease: reliability of sleep questionnaires compared to polysomnography. Neurodegener Dis. 2017;17(6):330–7. https://doi.org/10.1159/000480701.

    Article  PubMed  Google Scholar 

  32. Diago EB, Martínez-Horta S, Lasaosa SS, Alebesque AV, Pérez-Pérez J, Kulisevsky J, Del Val JL. Circadian rhythm, cognition, and mood disorders in Huntington’s disease. J Huntingtons Dis. 2018;7(2):193–8. https://doi.org/10.3233/JHD-180291.

    Article  PubMed  Google Scholar 

  33. Tanigaki WK, Rossetti MA, Rocha NP, Stimming EF. Sleep dysfunction in Huntington’s disease: perspectives from patients. J Huntingtons Dis. 2020;9(4):345–52. https://doi.org/10.3233/JHD-200434.

    Article  PubMed  Google Scholar 

  34. Goodman AO, Rogers L, Pilsworth S, McAllister CJ, Shneerson JM, Morton AJ, Barker RA. Asymptomatic sleep abnormalities are a common early feature in patients with Huntington’s disease. Curr Neurol Neurosci Rep. 2011;11(2):211–7. https://doi.org/10.1007/s11910-010-0163-x.

    Article  PubMed  Google Scholar 

  35. Piano C, Losurdo A, Della Marca G, Solito M, Calandra-Buonaura G, Provini F, Bentivoglio AR, Cortelli P. Polysomnographic findings and clinical correlates in Huntington disease: a cross-sectional cohort study. Sleep. 2015;38(9):1489–95. https://doi.org/10.5665/sleep.4996.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Evers S, Stögbauer F. Genetic association of Huntington’s disease and restless legs syndrome? A family report. Mov Disord. 2003;18(2):225–7. https://doi.org/10.1002/mds.10341.

    Article  PubMed  Google Scholar 

  37. Savva E, Schnorf H, Burkhard PR. Restless legs syndrome: an early manifestation of Huntington’s disease? Acta Neurol Scand. 2009;119(4):274–6. https://doi.org/10.1111/j.1600-0404.2008.01091.x.

    Article  CAS  PubMed  Google Scholar 

  38. Banno K, Hobson DE, Kryger MH. Long-term treatment of sleep breathing disorder in a patient with Huntington’s disease. Parkinsonism Relat Disord. 2005;11(4):261–4. https://doi.org/10.1016/j.parkreldis.2005.01.005.

    Article  PubMed  Google Scholar 

  39. Bollen EL, Den Heijer JC, Ponsioen C, Kramer C, Van der Velde EA, Van Dijk JG, Roos RA, Kamphuisen HA, Buruma OJ. Respiration during sleep in Huntington’s chorea. J Neurol Sci. 1988;84(1):63–8. https://doi.org/10.1016/0022-510x(88)90174-8.

    Article  CAS  PubMed  Google Scholar 

  40. Wiegand M, Möller AA, Schreiber W, Lauer C, Krieg JC. Brain morphology and sleep EEG in patients with Huntington’s disease. Eur Arch Psychiatry Clin Neurosci. 1991;240(3):148–52. https://doi.org/10.1007/BF02190755.

    Article  CAS  PubMed  Google Scholar 

  41. Silvestri R, Raffaele M, De Domenico P, Tisano A, Mento G, Casella C, Tripoli MC, Serra S, Di Perri R. Sleep features in Tourette’s syndrome, neuroacanthocytosis and Huntington’s chorea. Neurophysiol Clin. 1995;25(2):66–77. https://doi.org/10.1016/0987-7053(96)81034-3.

    Article  CAS  PubMed  Google Scholar 

  42. Cuturic M, Abramson RK, Vallini D, Frank EM, Shamsnia M. Sleep patterns in patients with Huntington’s disease and their unaffected first-degree relatives: a brief report. Behav Sleep Med. 2009;7(4):245–54. https://doi.org/10.1080/15402000903190215.

    Article  PubMed  Google Scholar 

  43. Marotta J, Piano C, Brunetti V, Genovese D, Bentivoglio AR, Calabresi P, Cortelli P, Della MG. Heart rate variability during wake and sleep in Huntington’s disease patients: an observational, cross-sectional, cohort study. Neurodegener Dis. 2021;21(3-4):79–86. https://doi.org/10.1159/000520754.

    Article  PubMed  Google Scholar 

  44. Annapureddy J, Ray S, Kamble N, Kumar G, Pal PK, Dv S, Jain S, Kutty B, Yadav R. The association of saccadic abnormalities with rem sleep in patients with Huntington’s disease. Sleep Med. 2022;93:84–9. https://doi.org/10.1016/j.sleep.2021.10.035.

    Article  PubMed  Google Scholar 

  45. •• Zhang Y, Ren R, Yang L, Zhou J, Li Y, Shi J, Lu L, Sanford LD, Tang X. Sleep in Huntington’s disease: a systematic review and meta-analysis of polysomnographic findings. Sleep. 2019;42(10):zsz154. https://doi.org/10.1093/sleep/zsz154. Meta-analysis of sleep disorderes and polysomnographic studies in Huntington’s disease.

    Article  PubMed  PubMed Central  Google Scholar 

  46. van Wamelen DJ, Aziz NA, Anink JJ, van Steenhoven R, Angeloni D, Fraschini F, Jockers R, Roos RA, Swaab DF. Suprachiasmatic nucleus neuropeptide expression in patients with Huntington’s disease. Sleep. 2013;36(1):117–25. https://doi.org/10.5665/sleep.2314.

    Article  PubMed  PubMed Central  Google Scholar 

  47. Kalliolia E, Silajdžić E, Nambron R, Hill NR, Doshi A, Frost C, Watt H, Hindmarsh P, Björkqvist M, Warner TT. Plasma melatonin is reduced in Huntington’s disease. Mov Disord. 2014;29(12):1511–5. https://doi.org/10.1002/mds.26003.

    Article  CAS  PubMed  Google Scholar 

  48. Aziz NA, Pijl H, Frölich M, Schröder-van der Elst JP, van der Bent C, Roelfsema F, Roos RA. Delayed onset of the diurnal melatonin rise in patients with Huntington’s disease. J Neurol. 2009;256(12):1961–5. https://doi.org/10.1007/s00415-009-5196-1.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Alders J, Smits M, Kremer B, Naarding P. The role of melatonin in sleep disturbances in end-stage Huntington’s disease. J Neuropsychiatry Clin Neurosci. 2009;21(2):226–7. https://doi.org/10.1176/jnp.2009.21.2.226.

    Article  PubMed  Google Scholar 

  50. Petersén A, Gil J, Maat-Schieman ML, Björkqvist M, Tanila H, Araújo IM, Smith R, Popovic N, Wierup N, Norlén P, Li JY, Roos RA, Sundler F, Mulder H, Brundin P. Orexin loss in Huntington’s disease. Hum Mol Genet. 2005;14(1):39–47. https://doi.org/10.1093/hmg/ddi004.

    Article  CAS  PubMed  Google Scholar 

  51. Meier A, Mollenhauer B, Cohrs S, Rodenbeck A, Jordan W, Meller J, Otto M. Normal hypocretin-1 (orexin-A) levels in the cerebrospinal fluid of patients with Huntington’s disease. Brain Res. 2005;1063(2):201–3.

    Article  CAS  PubMed  Google Scholar 

  52. Kudo T, Schroeder A, Loh DH, Kuljis D, Jordan MC, Roos KP, Colwell CS. Dysfunctions in circadian behavior and physiology in mouse models of Huntington’s disease. Exp Neurol. 2011;228(1):80–90. https://doi.org/10.1016/j.expneurol.2010.12.011.

    Article  CAS  PubMed  Google Scholar 

  53. Pallier PN, Morton AJ. Management of sleep/wake cycles improves cognitive function in a transgenic mouse model of Huntington's disease. Brain Res. 2009;1279:90–8. https://doi.org/10.1016/j.brainres.2009.03.072.

    Article  CAS  PubMed  Google Scholar 

  54. Wood NI, McAllister CJ, Cuesta M, Aungier J, Fraenkel E, Morton AJ. Adaptation to experimental jet-lag in R6/2 mice despite circadian dysrhythmia. PLoS One. 2013;8(2):e55036. https://doi.org/10.1371/journal.pone.0055036.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  55. Kantor S, Szabo L, Varga J, Cuesta M, Morton AJ. Progressive sleep and electroencephalogram changes in mice carrying the Huntington’s disease mutation. Brain. 2013;136(Pt 7):2147–58. https://doi.org/10.1093/brain/awt128.

    Article  PubMed  Google Scholar 

  56. Fisher SP, Black SW, Schwartz MD, Wilk AJ, Chen TM, Lincoln WU, Liu HW, Kilduff TS, Morairty SR. Longitudinal analysis of the electroencephalogram and sleep phenotype in the R6/2 mouse model of Huntington's disease. Brain. 2013;136(Pt 7):2159–72.

    Article  PubMed  Google Scholar 

  57. Lebreton F, Cayzac S, Pietropaolo S, Jeantet Y, Cho YH. Sleep physiology alterations precede plethoric phenotypic changes in R6/1 Huntington’s disease mice. PLoS One. 2015;10(5):e0126972. https://doi.org/10.1371/journal.pone.0126972.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  58. Loh DH, Kudo T, Truong D, Wu Y, Colwell CS. The Q175 mouse model of Huntington’s disease shows gene dosage- and age-related decline in circadian rhythms of activity and sleep. PLoS One. 2013;8(7):e69993. https://doi.org/10.1371/journal.pone.0069993.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  59. Fisher SP, Schwartz MD, Wurts-Black S, Thomas AM, Chen TM, Miller MA, Palmerston JB, Kilduff TS, Morairty SR. Quantitative electroencephalographic analysis provides an early-stage indicator of disease onset and progression in the zQ175 knock-in mouse model of Huntington’s disease. Sleep. 2016;39(2):379–91. https://doi.org/10.5665/sleep.5448.

    Article  PubMed  PubMed Central  Google Scholar 

  60. Kudo T, Loh DH, Tahara Y, Truong D, Hernández-Echeagaray E, Colwell CS. Circadian dysfunction in response to in vivo treatment with the mitochondrial toxin 3-nitropropionic acid. ASN Neuro. 2014;6(1):e00133. https://doi.org/10.1042/AN20130042.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  61. Morton AJ, Rudiger SR, Wood NI, Sawiak SJ, Brown GC, Mclaughlan CJ, Kuchel TR, Snell RG, Faull RL, Bawden CS. Early and progressive circadian abnormalities in Huntington’s disease sheep are unmasked by social environment. Hum Mol Genet. 2014;23(13):3375–83. https://doi.org/10.1093/hmg/ddu047.

    Article  CAS  PubMed  Google Scholar 

  62. Vas S, Nicol AU, Kalmar L, Miles J, Morton AJ. Abnormal patterns of sleep and EEG power distribution during non-rapid eye movement sleep in the sheep model of Huntington’s disease. Neurobiol Dis. 2021;155:105367. https://doi.org/10.1016/j.nbd.2021.105367.

    Article  CAS  PubMed  Google Scholar 

  63. Gonzales ED, Tanenhaus AK, Zhang J, Chaffee RP, Yin JC. Early-onset sleep defects in Drosophila models of Huntington’s disease reflect alterations of PKA/CREB signaling. Hum Mol Genet. 2016;25(5):837–52. https://doi.org/10.1093/hmg/ddv482.

    Article  CAS  PubMed  Google Scholar 

  64. Cabanas M, Pistono C, Puygrenier L, Rakesh D, Jeantet Y, Garret M, Cho YH. Neurophysiological and behavioral effects of anti-orexinergic treatments in a mouse model of Huntington’s disease. Neurotherapeutics. 2019;16(3):784–96. https://doi.org/10.1007/s13311-019-00726-3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  65. Shan L, Swaab DF. Changes in histaminergic system in neuropsychiatric disorders and the potential treatment consequences. Curr Neuropharmacol. 2022;20(2):403–11. https://doi.org/10.2174/1570159X19666210909144930.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  66. Fisher RH, Norris JW, DeManuele F, Ridgley B, Malyon C. Huntington’s chorea. Can Med Assoc J. 1982;126(6):605–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  67. Anderson KE, van Duijn E, Craufurd D, Drazinic C, Edmondson M, Goodman N, van Kammen D, Loy C, Priller J, Goodman LV. Clinical management of neuropsychiatric symptoms of Huntington disease: expert-based consensus guidelines on agitation, anxiety, apathy, psychosis and sleep disorders. J Huntingtons Dis. 2018;7(3):355–66. https://doi.org/10.3233/JHD-180293.

    Article  PubMed  PubMed Central  Google Scholar 

  68. Lucena-Valera A, Ruz-Zafra P, Ampuero J. Wilson’s disease: overview. Med Clin (Barc). 2023;23:261–7. https://doi.org/10.1016/j.medcli.2022.12.016.

    Article  CAS  Google Scholar 

  69. Soltanzadeh A, Soltanzadeh P, Nafissi S, Ghorbani A, Sikaroodi H, Lotfi J. Wilson’s disease: a great masquerader. Eur Neurol. 2007;57(2):80–5. https://doi.org/10.1159/000098056.

    Article  PubMed  Google Scholar 

  70. Nevsimalova S, Buskova J, Bruha R, Kemlink D, Sonka K, Vitek L, Marecek Z. Sleep disorders in Wilson’s disease. Eur J Neurol. 2011;18(1):184–90. https://doi.org/10.1111/j.1468-1331.2010.03106.x.

    Article  CAS  PubMed  Google Scholar 

  71. Portala K, Westermark K, von Knorring L, Ekselius L. Psychopathology in treated Wilson’s disease determined by means of CPRS expert and self-ratings. Acta Psychiatr Scand. 2000;101(2):104–9. https://doi.org/10.1034/j.1600-0447.2000.90085.x.

    Article  CAS  PubMed  Google Scholar 

  72. Portala K, Westermark K, Ekselius L, Broman JE. Sleep in patients with treated Wilson’s disease. A questionnaire study. Nord J Psychiatry. 2002;56(4):291–7. https://doi.org/10.1080/08039480260242796.

    Article  PubMed  Google Scholar 

  73. Cochen De Cock V, Lacombe S, Woimant F, Poujois A. Sleep disorders in Wilson’s disease. Sleep Med. 2021;83:299–303. https://doi.org/10.1016/j.sleep.2020.07.007.

    Article  PubMed  Google Scholar 

  74. Jernajczyk W, Litwin T, Członkowska A, Bembenek JP. Sleep disturbances in newly diagnosed treatment-naïve patients with Wilson’s disease. Acta Neurol Belg. 2022;122(3):745–51. https://doi.org/10.1007/s13760-022-01915-2.

    Article  PubMed  PubMed Central  Google Scholar 

  75. Chakraborty AP, Mukherjee A, Sarkar S, Sinharoy U, Biswas A. Evaluation of non-motor symptoms in Wilson disease using the Parkinson’s disease nonmotor symptoms questionnaire: a pilot cross-sectional study and critical assessment. Ann Indian Acad Neurol. 2022;25:1062–6.

    PubMed  PubMed Central  Google Scholar 

  76. Netto AB, Sinha S, Taly AB, Panda S, Rao S. Sleep in Wilson’s disease: questionnaire based study. Ann Indian Acad Neurol. 2011;14(1):31–4. https://doi.org/10.4103/0972-2327.78047.

    Article  PubMed  PubMed Central  Google Scholar 

  77. Tribl GG, Trindade MC, Bittencourt T, Lorenzi-Filho G, Cardoso Alves R, Ciampi de Andrade D, Fonoff ET, Bor-Seng-Shu E, Machado AA, Schenck CH, Teixeira MJ, Barbosa ER. Wilson’s disease with and without rapid eye movement sleep behavior disorder compared to healthy matched controls. Sleep Med. 2016;17:179–85. https://doi.org/10.1016/j.sleep.2015.09.003.

    Article  PubMed  Google Scholar 

  78. You Z, Xu H, Wu Z, You Z. Sleep disorders in Wilson’s disease: a questionnaire study. Neurol Sci. 2023;44(1):209–14. https://doi.org/10.1007/s10072-022-06401-4.

    Article  PubMed  Google Scholar 

  79. Palmieri GR, De Michele G, Matarazzo M, Di Dato F, Perillo S, Dello Iacovo DCP, Cuomo N, Pane C, Russo CV, Iorio R, De Michele G, De Rosa A. Prevalence and features of non-motor symptoms in Wilson’s disease. Parkinsonism Relat Disord. 2022;95:103–6. https://doi.org/10.1016/j.parkreldis.2022.01.016.

    Article  PubMed  Google Scholar 

  80. Firneisz G, Szalay F, Halasz P, Komoly S. Hypersomnia in Wilson’s disease: an unusual symptom in an unusual case. Acta Neurol Scand. 2000;101(4):286–8. https://doi.org/10.1034/j.1600-0404.2000.101004286.x.

    Article  CAS  PubMed  Google Scholar 

  81. Amann VC, Maru NK, Jain V. Hypersomnolence in Wilson disease. J Clin Sleep Med. 2015;11:1341–3. https://doi.org/10.5664/jcsm.5204.

    Article  PubMed  PubMed Central  Google Scholar 

  82. • Trindade MC, Bittencourt T, Lorenzi-Filho G, Alves RC, de Andrade DC, Fonoff ET, Bor-Seng-Shu E, Machado AA, Teixeira MJ, Barbosa ER, Tribl GG. Restless legs syndrome in Wilson’s disease: frequency, characteristics, and mimics. Acta Neurol Scand. 2017;135(2):211–8. https://doi.org/10.1111/ane.12585. Association between restless legs syndrome and Wilson’s disease.

    Article  CAS  PubMed  Google Scholar 

  83. • Tribl GG, Bor-Seng-Shu E, Trindade MC, Lucato LT, Teixeira MJ, Barbosa ER. Wilson’s disease presenting as rapid eye movement sleep behavior disorder: a possible window to early treatment. Arq Neuropsiquiatr. 2014;72(9):653–8. https://doi.org/10.1590/0004-282x20140118. Association between REM sleep behavior disorder and Wilson’s disease.

    Article  PubMed  Google Scholar 

  84. Voloshyna D, Bseiso A, Shaik TA, Sedhai S, Abdelsamad A, Khan MH, Sandhu QI, Saleem F, Ghaffari MAZ. Rapid eye movement behavior sleep disorder as a primary manifestation of Wilson disease: a gateway to early intervention. Cureus. 2022;14(9):e29618. https://doi.org/10.7759/cureus.29618.

    Article  PubMed  PubMed Central  Google Scholar 

  85. Keir LHM, Breen DP. REM sleep behaviour disorder in patients without synucleinopathy. J Neurol Neurosurg Psychiatry. 2020;91(11):1239–40. https://doi.org/10.1136/jnnp-2020-323475.

    Article  PubMed  Google Scholar 

  86. Zhang S, Li L, Wang J. Wilson disease patient with rare heterozygous mutations in ATP7B accompanied by distinctive nocturnal enuresis: a case report. Medicine (Baltimore). 2020;99(28):e20997. https://doi.org/10.1097/MD.0000000000020997.

    Article  PubMed  PubMed Central  Google Scholar 

  87. Netto AB, Sinha S, Taly AB, Panda S, Rao S. Sleep in Wilson’s disease: a polysomnography-based study. Neurol India. 2010;58(6):933–8. https://doi.org/10.4103/0028-3886.73752.

    Article  PubMed  Google Scholar 

  88. Cochen De Cock V, Girardot-Tinant N, Woimant F, Poujois A. Sleep abnormalities in Wilson’s disease. Curr Treat Options Neurol. 2018;20(11):46. https://doi.org/10.1007/s11940-018-0531-4.

    Article  PubMed  Google Scholar 

  89. Seow SY, Kwok KFV, Tay KH, Chee WSA, Rawtaer I, Cheng Y, Tan QX, Tan SM. Systematic review of clinical practice guidelines for insomnia disorder. J Psychiatr Pract. 2022;28(6):465–77. https://doi.org/10.1097/PRA.0000000000000670.

    Article  PubMed  Google Scholar 

  90. Gossard TR, Trotti LM, Videnovic A, St Louis EK. Restless legs syndrome: contemporary diagnosis and treatment. Neurotherapeutics. 2021;18(1):140–55. https://doi.org/10.1007/s13311-021-01019-4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  91. Jiménez-Jiménez FJ, Alonso-Navarro H, García-Martín E, Agúndez JAG. Current treatment options for REM sleep behaviour disorder. J Pers Med. 2021;11(11):1204. https://doi.org/10.3390/jpm11111204.

    Article  PubMed  PubMed Central  Google Scholar 

  92. Peikert K, Danek A, Hermann A. Current state of knowledge in chorea-acanthocytosis as core neuroacanthocytosis syndrome. Eur J Med Genet. 2018;61(11):699–705. https://doi.org/10.1016/j.ejmg.2017.12.007.

    Article  PubMed  Google Scholar 

  93. Hori A, Kazukawa S, Nakamura I, Endo M. Electroencephalographic findings in neuroacanthocytosis. Electroencephalogr Clin Neurophysiol. 1985;61(5):342–8. https://doi.org/10.1016/0013-4694(85)91024-7.

    Article  CAS  PubMed  Google Scholar 

  94. Bramanti P, Ricci RM, Candela L, Di Bella P, Serra S, Xerra A, Di Perri R. Sleep spindles in amyotrophic chorea-acanthocytosis disease. Acta Neurol (Napoli). 1987 Jun;9(3):191–8.

    CAS  PubMed  Google Scholar 

  95. Weaver J, Sarva H, Barone D, Bobker S, Bushara K, Hiller A, Ishii M, Jankovic J, Lakhani S, Niotis K, Scharre DW, Tuite P, Stutz A, Westhoff CM, Walker RH. McLeod syndrome: five new pedigrees with novel mutations. Parkinsonism Relat Disord. 2019;64:293–9. https://doi.org/10.1016/j.parkreldis.2019.04.022.

    Article  CAS  PubMed  Google Scholar 

  96. Lim MM, Sarva H, Hiller A, Feitell S, Oates P, Barone D, Walker RH. Sleep disorders in McLeod syndrome: a case series. Parkinsonism Relat Disord. 2022;102:86–8. https://doi.org/10.1016/j.parkreldis.2022.07.013.

    Article  PubMed  Google Scholar 

  97. Hernández Vara J, Fernández Cortijo J, Purroy García F, Miquel Rodríguez F. Síndrome de piernas inquietas y neuroacantocitosis [Restless legs syndrome and neuroacanthocytosis]. Med Clin (Barc). 2005;124(18):717–8. https://doi.org/10.1157/13075104.

    Article  PubMed  Google Scholar 

  98. •• Sabater L, Gaig C, Gelpi E, Bataller L, Lewerenz J, Torres-Vega E, Contreras A, Giometto B, Compta Y, Embid C, Vilaseca I, Iranzo A, Santamaría J, Dalmau J, Graus F. A novel non-rapid-eye movement and rapid-eye-movement parasomnia with sleep breathing disorder associated with antibodies to IgLON5: a case series, characterisation of the antigen, and post-mortem study. Lancet Neurol. 2014;13(6):575–86. https://doi.org/10.1016/S1474-4422(14)70051-1. Description of a new syndrome associating sleep disorders and chorea.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  99. Simabukuro MM, Sabater L, Adoni T, Cury RG, Haddad MS, Moreira CH, Oliveira L, Boaventura M, Alves RC, Azevedo Soster L, Nitrini R, Gaig C, Santamaria J, Dalmau J, Graus F. Sleep disorder, chorea, and dementia associated with IgLON5 antibodies. Neurol Neuroimmunol Neuroinflamm. 2015;2(4):e136. https://doi.org/10.1212/NXI.0000000000000136.

    Article  PubMed  PubMed Central  Google Scholar 

  100. Haitao R, Yingmai Y, Yan H, Fei H, Xia L, Honglin H, Chaiyan L, Stöcker W, Liying C, Hongzhi G. Chorea and parkinsonism associated with autoantibodies to IgLON5 and responsive to immunotherapy. J Neuroimmunol. 2016;15(300):9–10. https://doi.org/10.1016/j.jneuroim.2016.09.012.

    Article  CAS  Google Scholar 

  101. Montagna M, Amir R, De Volder I, Lammens M, Huyskens J, Willekens B. IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes. Front Neurol. 2018;17(9):329. https://doi.org/10.3389/fneur.2018.00329.

    Article  Google Scholar 

  102. Gaig C, Graus F, Compta Y, Högl B, Bataller L, Brüggemann N, Giordana C, Heidbreder A, Kotschet K, Lewerenz J, Macher S, Martí MJ, Montojo T, Pérez-Pérez J, Puertas I, Seitz C, Simabukuro M, Téllez N, Wandinger KP, et al. Clinical manifestations of the anti-IgLON5 disease. Neurology. 2017;88(18):1736–43. https://doi.org/10.1212/WNL.0000000000003887.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  103. • Gaig C, Compta Y, Heidbreder A, Marti MJ, Titulaer MJ, Crijnen Y, Högl B, Lewerenz J, Erro ME, Garcia-Monco JC, Nigro P, Tambasco N, Patalong-Ogiewa M, Erdler M, Macher S, Berger-Sieczkowski E, Höftberger R, Geis C, Hutterer M, et al. Frequency and characterization of movement disorders in anti-IgLON5 disease. Neurology. 2021;97(14):e1367–81. https://doi.org/10.1212/WNL.0000000000012639. Description of the clinical features of a new syndrome associating sleep disorders and chorea.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  104. Díaz-Grez F, Lay-Son L, del Barrio-Guerrero E, Vidal-González P, de Sydenham C. Análisis clinico de 55 pacientes con seguimiento prolongado [Sydenham’s chorea. A clinical analysis of 55 patients with a prolonged follow-up]. Rev Neurol. 2004;39(9):810–5.

    PubMed  Google Scholar 

  105. Ramm-Pettersen A, Nakken KO, Haavardsholm KC, Selmer KK. GLUT1-deficiency syndrome: report of a four-generation Norwegian family with a mild phenotype. Epilepsy Behav. 2017;70(Pt A):1–4. https://doi.org/10.1016/j.yebeh.2017.02.016.

    Article  PubMed  Google Scholar 

  106. Anurat K, Khongkhatithum C, Tim-Aroon T, Limwongse C, Thampratankul L. Sleep disorder: an overlooked manifestation of glucose transporter type-1 deficiency syndrome. Neuropediatrics. 2022;53(2):129–32. https://doi.org/10.1055/s-0041-1736179.

    Article  PubMed  Google Scholar 

  107. Iodice A, Carecchio M, Zorzi G, Garavaglia B, Spagnoli C, Salerno GG, Frattini D, Mencacci NE, Invernizzi F, Veneziano L, Mantuano E, Angriman M, Fusco C. Restless legs syndrome in NKX2-1-related chorea: an expansion of the disease spectrum. Brain Dev. 2019;41(3):250–6. https://doi.org/10.1016/j.braindev.2018.10.001.

    Article  CAS  PubMed  Google Scholar 

  108. Méneret A, Roze E, Maranci JB, Dodet P, Doummar D, Riant F, Tranchant C, Fraix V, Anheim M, Ekmen A, McGovern E, Vidailhet M, Arnulf I, Leu-Semenescu S. Sleep in ADCY5-related dyskinesia: prolonged awakenings caused by abnormal movements. J Clin Sleep Med. 2019;15(7):1021–9. https://doi.org/10.5664/jcsm.7886.

    Article  PubMed  PubMed Central  Google Scholar 

  109. Piard J, Béreau M, XiangWei W, Wirth T, Amsallem D, Buisson L, Richard P, Liu N, Xu Y, Myers SJ, Traynelis SF, Chelly J, Anheim M, Raynaud M, Maldergem LV, Yuan H. The GRIA3 c.2477G > A variant causes an exaggerated startle reflex, chorea, and multifocal myoclonus. Mov Disord. 2020;35(7):1224–32. https://doi.org/10.1002/mds.28058.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Funding

The work at the authors’ laboratory is supported in part by Grants PI18/00540 and PI21/01683 from Fondo de Investigación Sanitaria, Instituto de Salud Carlos III, Madrid, Spain, and IB20134 and GR21073 from Junta de Extremadura, Mérida, Spain. Partially funded with FEDER funds.

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  1. Section of Neurology, Hospital Universitario del Sureste, Ronda del Sur 10 E-28500, Arganda del Rey, Madrid, Spain

    Félix Javier Jiménez-Jiménez & Hortensia Alonso-Navarro

  2. Universidad de Extremadura, University Institute of Molecular Pathology Biomarkers, Cáceres, Spain

    Elena García-Martín & José A.G. Agúndez

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Jiménez-Jiménez, F.J., Alonso-Navarro, H., García-Martín, E. et al. Sleep Disorders in Patients with Choreic Syndromes. Curr Neurol Neurosci Rep 23, 361–379 (2023). https://doi.org/10.1007/s11910-023-01274-2

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